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Main epilepsy syndromes and related issues in children
S10
15th May 2008 (Thursday), Oral presentations 16.45 18.15
Can the outcome be predicted? The primary goal of the treatment is improved mental outcome. We know the long-term outcome after ACTH therapy, but not after VGB. Response to treatment seems to be of early predictive value. In the UK study the cognitive outlook evaluated at the age of 14 months was much improved for children who respond early to hormone treatment, in cryptogenic group. Can we make the prognosis better? The structural abnormalities strongly influence the prognosis. In 5 retrospective studies a short treatment lag (<4 weeks) correlated with a favourable outcome, in up to 100% in the cryptogenic group. a) Treat early! b) Avoid the side-effects of therapy. c) Some small etiological groups might be prevented. Favourable factors are the following: cryptogenic etiology, age at onset 4 months, absence of atypical spasms, partial seizures and asymmetrical EEG abnormalities, short treatment lag, and early and sustained response to treatment.
problems, it is not possible to predict whether educational delay may occur in an individual child with epilepsy. The effect of two factors that cause most concern in clinical practice will be addressed. ◦ Short and difficult-to-detect seizures and their effect on cognition and educational attainment, together with the peri- and postictal effects of such seizures and whether episodes with epileptiform EEG discharges (but without epileptic seizures) are associated with learning difficulties. ◦ The impact of central side effects of antiepileptic drug treatment. Such side effects can interfere with both cognition and mood and may precipitate problems in school. A further group of children who require special attention are those with epilepsy and co-morbid attention deficit hyperactivity disorder.
16.45 18.15 Satellite symposium: Update on pharmacological treatment of epilepsy UCB01 Main epilepsy syndromes and related issues in children J.H. Cross. UCL-Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, London, England, UK Epilepsy remains the most common ongoing neurological disorder in children. Misdiagnosis rates remain high, even amongst those attending a tertiary referral centre. A correct diagnosis of epilepsy would seem to be paramount, but in addition the diagnosis of the correct type of epilepsy is as important. Epilepsy syndromes are clusters of symptoms and signs that can be recognised and readily identified, which may include evidence from clinical (e.g. history, seizure type), neurophysiological and neuroradiological investigations. The majority have onset in childhood, and definition of such enables plans to be made for optimal management, as well as an ability to outline a likely prognosis. An original classification of epilepsy syndromes was proposed by the International League Against Epilepsy (ILAE) in 1990; this was subsequently revised in 2001 in view of the increasing number of syndromes identified and some ambiguity in terminology. The original classification also had disadvantages with regard to the fact it did not take into account any underlying pathophysiology or associate co-morbidities. The current classification is based on a five axis system to aid diagnosis, from whether epilepsy is the diagnosis through to associated co-morbidities seen. The diagnosis of individual syndromes has enabled wider study of pathophysiological correlations, in particular the phenotypes associated with specific structural brain malformations. It has also helped give further insights into the genetic basis of the epilepsies. The rate of behavioural and cognitive co-morbidities in children with epilepsy remains high, in some readily recognised as part of the symptom cluster of the syndrome. Ongoing study is required to determine whether the natural history of these may be influenced by early intervention. UCB02 The child with epilepsy at school A. Aldenkamp. University of Maastricht, Maastricht University Hospital, The Netherlands Children with epilepsy have a greater risk than their peers of developing co-morbid learning difficulties. As it is unknown which factors contribute to the development of such learning
Reference(s) Aldenkamp AP, Arzimanoglou A, Reijs R, Van Mil S. Optimizing therapy of seizures in children and adolescents with ADHD. Neurology 2006; 67(S4): S49−51. Aldenkamp AP, Arends J. The relative influence of epileptic EEG discharges, short nonconvulsive seizures and type of epilepsy on cognitive function. Epilepsia 2004; 45: 54−63. Tromp S, Aldenkamp AP, Arends J, Weber JW, van der Linden I, Diepman L. Relative influence of epileptic seizures and of epilepsy syndrome on cognitive function. J Child Neurol 2003; 18: 407−13. Aldenkamp AP, De Krom M, Reijs R. Newer antiepileptic drugs and cognitive issues. Epilepsia 2003; 44(S4): 21−9.
UCB03 Anti-epileptic drugs: efficacy and spectrum of action in children L. Lagae. University of Leuven, KUL University Hospitals, Leuven, Belgium The choice of an optimal anti-epileptic drug (AED) in a child with epilepsy depends on several different factors. That the drug is effective against the epileptic seizures is of primary importance. Theoretically, efficacy could be deduced from a thorough understanding of the underlying pathophysiological mechanisms of the epilepsy and the working mechanism(s) of the AED. In practice, however, clinicians are guided by the results of randomised controlled trials, guidelines and expert opinions. Other equally important parameters in the choice of AED are the safety profile of the drug and the individual characteristics of the child such as age, sex and co-morbidities. Therefore, every treatment choice should be tailored to the individual child. As the profiles of the existing AEDs are well known, it seems straightforward to use those that are effective against the most common seizure types seen in childhood epilepsy. In the paediatric population, however, broad-spectrum AEDs (e.g. valproate, topiramate, levetiracetam, lamotrigine) are very useful. Commencing treatment with a safe broadspectrum AED is a valuable option, especially in infants and young children in whom the exact seizure type (primary generalised or partial) is not always initially clear. In the more severe childhood epilepsy syndromes, both generalised and partial seizures are seen and here also, broad-spectrum AEDs are helpful. In recent years it has become clear that some AEDs can aggravate epilepsy and are to be avoided in some epilepsy syndromes. Clinical experience is of particular use in guiding the choice of a rational polytherapy scheme in refractory cases. An optimal choice of AED thus still depends on accurate characterisation of the epilepsy syndrome, and the individual characteristics of the child. Reference(s) Lagae L. The need for broad spectrum and safe anti-epileptic drugs in childhood epilepsy. European Neurological Disease 2006; 2: 36−9. Available at http://www.touchbriefings.com/download.cfm?fileID= 10428
15th May 2008 (Thursday), Oral presentations 16.45 18.15
Can the outcome be predicted? The primary goal of the treatment is improved mental outcome. We know the long-term outcome after ACTH therapy, but not after VGB. Response to treatment seems to be of early predictive value. In the UK study the cognitive outlook evaluated at the age of 14 months was much improved for children who respond early to hormone treatment, in cryptogenic group. Can we make the prognosis better? The structural abnormalities strongly influence the prognosis. In 5 retrospective studies a short treatment lag (<4 weeks) correlated with a favourable outcome, in up to 100% in the cryptogenic group. a) Treat early! b) Avoid the side-effects of therapy. c) Some small etiological groups might be prevented. Favourable factors are the following: cryptogenic etiology, age at onset 4 months, absence of atypical spasms, partial seizures and asymmetrical EEG abnormalities, short treatment lag, and early and sustained response to treatment.
problems, it is not possible to predict whether educational delay may occur in an individual child with epilepsy. The effect of two factors that cause most concern in clinical practice will be addressed. ◦ Short and difficult-to-detect seizures and their effect on cognition and educational attainment, together with the peri- and postictal effects of such seizures and whether episodes with epileptiform EEG discharges (but without epileptic seizures) are associated with learning difficulties. ◦ The impact of central side effects of antiepileptic drug treatment. Such side effects can interfere with both cognition and mood and may precipitate problems in school. A further group of children who require special attention are those with epilepsy and co-morbid attention deficit hyperactivity disorder.
16.45 18.15 Satellite symposium: Update on pharmacological treatment of epilepsy UCB01 Main epilepsy syndromes and related issues in children J.H. Cross. UCL-Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, London, England, UK Epilepsy remains the most common ongoing neurological disorder in children. Misdiagnosis rates remain high, even amongst those attending a tertiary referral centre. A correct diagnosis of epilepsy would seem to be paramount, but in addition the diagnosis of the correct type of epilepsy is as important. Epilepsy syndromes are clusters of symptoms and signs that can be recognised and readily identified, which may include evidence from clinical (e.g. history, seizure type), neurophysiological and neuroradiological investigations. The majority have onset in childhood, and definition of such enables plans to be made for optimal management, as well as an ability to outline a likely prognosis. An original classification of epilepsy syndromes was proposed by the International League Against Epilepsy (ILAE) in 1990; this was subsequently revised in 2001 in view of the increasing number of syndromes identified and some ambiguity in terminology. The original classification also had disadvantages with regard to the fact it did not take into account any underlying pathophysiology or associate co-morbidities. The current classification is based on a five axis system to aid diagnosis, from whether epilepsy is the diagnosis through to associated co-morbidities seen. The diagnosis of individual syndromes has enabled wider study of pathophysiological correlations, in particular the phenotypes associated with specific structural brain malformations. It has also helped give further insights into the genetic basis of the epilepsies. The rate of behavioural and cognitive co-morbidities in children with epilepsy remains high, in some readily recognised as part of the symptom cluster of the syndrome. Ongoing study is required to determine whether the natural history of these may be influenced by early intervention. UCB02 The child with epilepsy at school A. Aldenkamp. University of Maastricht, Maastricht University Hospital, The Netherlands Children with epilepsy have a greater risk than their peers of developing co-morbid learning difficulties. As it is unknown which factors contribute to the development of such learning
Reference(s) Aldenkamp AP, Arzimanoglou A, Reijs R, Van Mil S. Optimizing therapy of seizures in children and adolescents with ADHD. Neurology 2006; 67(S4): S49−51. Aldenkamp AP, Arends J. The relative influence of epileptic EEG discharges, short nonconvulsive seizures and type of epilepsy on cognitive function. Epilepsia 2004; 45: 54−63. Tromp S, Aldenkamp AP, Arends J, Weber JW, van der Linden I, Diepman L. Relative influence of epileptic seizures and of epilepsy syndrome on cognitive function. J Child Neurol 2003; 18: 407−13. Aldenkamp AP, De Krom M, Reijs R. Newer antiepileptic drugs and cognitive issues. Epilepsia 2003; 44(S4): 21−9.
UCB03 Anti-epileptic drugs: efficacy and spectrum of action in children L. Lagae. University of Leuven, KUL University Hospitals, Leuven, Belgium The choice of an optimal anti-epileptic drug (AED) in a child with epilepsy depends on several different factors. That the drug is effective against the epileptic seizures is of primary importance. Theoretically, efficacy could be deduced from a thorough understanding of the underlying pathophysiological mechanisms of the epilepsy and the working mechanism(s) of the AED. In practice, however, clinicians are guided by the results of randomised controlled trials, guidelines and expert opinions. Other equally important parameters in the choice of AED are the safety profile of the drug and the individual characteristics of the child such as age, sex and co-morbidities. Therefore, every treatment choice should be tailored to the individual child. As the profiles of the existing AEDs are well known, it seems straightforward to use those that are effective against the most common seizure types seen in childhood epilepsy. In the paediatric population, however, broad-spectrum AEDs (e.g. valproate, topiramate, levetiracetam, lamotrigine) are very useful. Commencing treatment with a safe broadspectrum AED is a valuable option, especially in infants and young children in whom the exact seizure type (primary generalised or partial) is not always initially clear. In the more severe childhood epilepsy syndromes, both generalised and partial seizures are seen and here also, broad-spectrum AEDs are helpful. In recent years it has become clear that some AEDs can aggravate epilepsy and are to be avoided in some epilepsy syndromes. Clinical experience is of particular use in guiding the choice of a rational polytherapy scheme in refractory cases. An optimal choice of AED thus still depends on accurate characterisation of the epilepsy syndrome, and the individual characteristics of the child. Reference(s) Lagae L. The need for broad spectrum and safe anti-epileptic drugs in childhood epilepsy. European Neurological Disease 2006; 2: 36−9. Available at http://www.touchbriefings.com/download.cfm?fileID= 10428